Vulvar Paget's Disease with an Underlying Sweat Gland Adenocarcinoma

Abstract

Vulvar Paget's disease, in contrast to its mammary counterpart, has been found to be associated with an underlying apocrine sweat gland carcinoma in about only 1/4 of the cases. There are two principal theories regarding the pathogenesis of extramammary Paget's disease: (1) intraepidermal origin of the Paget cells occurring independently of, or concomitantly with, an underlying apocrine gland carcinoma, and (2) the migration or metastasis of Paget cells into the epidermis from the underlying apocrine glands and/or ducts. A case of vulvar Paget's disease that, in our opinion, supports the theory of apocrine gland origin of Paget cells is reported herein.

Treatment Outcomes of Adjunct and Stand-alone Photodynamic Therapy in Patients With Extramammary Paget Disease: A Literature Review

“…5 The first theory proposes that the intraepidermal origin of Paget cells have the potential to be associated with an apocrine gland carcinoma; the second theory proposes that it arises from the metastasis of Paget cells into the epidermis from the underlying apocrine glands. 6 The exact incidence of EMPD is not known but is estimated to account for 1% to 6% of all Paget disease cases. 7 Management of EMPD is challenging due to the multifocal presentation and lack of correlation between gross and microscopic borders of the lesion.…”